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Severe Type of Neonatal Intrahepatic Cholestatic Jaundice by Citrin Deficiency (씨트린 결핍증에 의한 중증의 신생아 담즙 정체성 활달)

  • Lee, Beom Hee;Kim, Yoo-Mi;Kim, Gu-Hwan;Yoo, Han-Wook
    • Journal of The Korean Society of Inherited Metabolic disease
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    • v.12 no.1
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    • pp.35-41
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    • 2012
  • Since the causative gene, SLC25A13 which encodes citrin, was discovered in 1999, over 500 cases with citrin deficiency have been identified. Two phenotypes can occur by citrin deficiency, neonatal intrahepatic cholestasis by citrin deficiency (NICCD) and adult-onset type II citrullinemia (CTLN2). Some patients with NICCD develop CTLN2 in their later lives. Although cholestatic jaundice is spontaneously resolved within the first year of life in most cases with NICCD, a few cases experience progressive hepatic failure. In this report, two neonates with severe type of NICCD were described. Both cases exhibited neonatal cholestatic jaundice, hyperammonemia and severe coagulopathy. Of note, plasma citrulline and blood galactose levels were extremely high. Serum ${\alpha}$-fetoprotein, plasma methionine, arginine, and threonine-to-serine ratio were elevated as well. SLC25A13 mutations were found in all the four alleles of both patients. With the commencement of lactose-free formula, coagulopathy and hyperammonemia were resolved, and galactose level was normalized. Currently, no factor has been identified to predict the prognosis of NICCD. More experiences are needed to build up the adequate therapeutic strategies for severe type of NICCD. Our experience, however, indicates that the degree of citrullinemia and galactosemia might reflect the severity.

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A Case of Transient Pseudohypoaldosteronism Secondary to Ureteropelvic Junction Obstruction (신우요관이행부폐색과 동반된 일과성 가성저알도스테론혈증 1례)

  • Choi Jun-Gi;Hahn Hye-Won;You Han-Wook;Park Young-Seo
    • Childhood Kidney Diseases
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    • v.8 no.1
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    • pp.91-95
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    • 2004
  • We report a 2-month-old boy who presented with severe hyponatremia and hyperkalemia secondary to ureteropelvic junction(VPJ) obstruction. By prenatal ultrasonography at 19 weeks of gestation, severe hydronephrosis was found which was confirmed postnatally Pyeloplasty was done on the 45th day of life, and fifteen days after pyeloplasty, non-bilious vomiting, decreased activity and dehydration developed. Severe hyponatremia and hyperkalemia were observed, as a result of elevated serum aldosterone and plasma renin activity. The anterior posterior pelvic diameter(APPD) and Society for Fetal Urology(SFU) grade measured showed no interval change before and after pyeloplasty. Pseudohypoaldosteronism was diagnosed, and 2M NaCl was administrated orally for 7 days. The electrolyte imbalance was corrected, and 8 weeks later, the elevated levels of aldosterone and plasma renin activity were normalized. The left hydronephrosis was improved at 5 months of age. We hereby report a transient pseudohypoaldosteronism secondary to UPJ obstruction with a review of the literature.

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Assessing the Extent and Rate of Deforestation in the Mountainous Tropical Forest

  • Pujiono, Eko;Lee, Woo-Kyun;Kwak, Doo-Ahn;Lee, Jong-Yeol
    • Korean Journal of Remote Sensing
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    • v.27 no.3
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    • pp.315-328
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    • 2011
  • Landsat data incorporated with additional bands-normalized difference vegetation index (NDVI) and band ratios were used to assess the extent and rate of deforestation in the Gunung Mutis Nature Reserve (GMNR), a mountainous tropical forest in Eastern of Indonesia. Hybrid classification was chosen as the classification approach. In this approach, the unsupervised classification-iterative self-organizing data analysis (ISODATA) was used to create signature files and training data set. A statistical separability measurement-transformed divergence (TD) was used to identify the combination of bands that showed the highest distinction between the land cover classes in training data set. Supervised classification-maximum likelihood classification (MLC) was performed using selected bands and the training data set. Post-classification smoothing and accuracy assessment were applied to classified image. Post-classification comparison was used to assess the extent of deforestation, of which the rate of deforestation was calculated by the formula suggested by Food Agriculture Organization (FAO). The results of two periods of deforestation assessment showed that the extent of deforestation during 1989-1999 was 720.72 ha, 0.80% of annual rate of deforestation, and its extent of deforestation during 1999-2009 was 1,059.12 ha, 1.31% of annual rate of deforestation. Such results are important for the GMNR authority to establish strategies, plans and actions for combating deforestation.

Spherical Indentation Techniques for Creep Property Evaluation Considering Transient Creep (천이크리프를 고려한 구형압입 크리프 물성평가법)

  • Lim, Dongkyu;Lee, Jin Haeng;Kim, Minsoo;Lee, Hyungyil
    • Transactions of the Korean Society of Mechanical Engineers A
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    • v.37 no.11
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    • pp.1339-1347
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    • 2013
  • Creep through nanoindentations has attracted increasing research attention in recent years. Many studies related to indentation creep tests, however, have simply focused on the characteristics of steady-state creep, and there exist wide discrepancies between the uniaxial test and the indentation test. In this study, we performed a computational simulation of spherical indentations, and we proposed a method for evaluating the creep properties considering transient creep. We investigated the material behavior with variation of creep properties and expressed it using regression equations for normalized variables. We finally developed a program to evaluate the creep properties considering transient creep. By using the proposed method, we successfully obtained creep exponents with an average error less than 1.1 and creep coefficients with an average error less than 2.3 from the load-depth curve.

Effect of Climate and Landscape Characteristics on Hydrologic Partitioning and Vegetation Response (기후와 지형 특성이 수문분할과 식생반응에 미치는 영향)

  • Park, Yoonkyung;Choi, Minha;Ahn, Jaehyun;Kim, Sangdan
    • Journal of Korea Water Resources Association
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    • v.46 no.7
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    • pp.735-744
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    • 2013
  • The effect of climate and landscape characteristics on hydrologic partitioning and vegetation response is analyzed in this study. After quantifying hydrologic partitioning using the Horton index, the relation between regional climate characteristics and the Horton index is investigated. In addition, using the comparison between the predictability of the Horton index with only regional climate characteristics and the predictability of the Horton index with landscape characteristics as well as regional climate characteristics, the relative contribution of landscape characteristics on hydrologic partitioning is analyzed. Finally, investigating the predictability of the aridity index and Horton index on the normalized difference vegetation index, the effect of climate and landscape characteristics on vegetation response is estimated.

Hydroacoustic Investigation of Demersal Fisheries Resources in the Southeastern Area of the Cheju Island , Korea - Acoustical Estimation of Fish Density and Distribution- (제주도 동남해역의 저서어업자원 조사연구 - 음향에 의한 어업생물의 분포밀도 추정 -)

  • Lee, Dae-Jae;Lee, Won-Woo
    • Journal of the Korean Society of Fisheries and Ocean Technology
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    • v.32 no.3
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    • pp.266-272
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    • 1996
  • The distribution and abundance of fish in the Cheju southeastern area was studied by the combined hydroacoustic and bottom trawl surveys in April 1994 and in July 1995, respectively. The main purpose of these investigations was to provide the basic data for this management and the biomass estimation of commercially important demersal fish stocks in this area. The hydroacoustic surveys were performed by using a 50 kHz scientific echo sounder system with a microcomputer-based echo integrator. Acoustical measurements of fish abundance and distribution were conducted along the cruise tracks of research vessel and during all trawl hauls by continuous echo sounding. The average weight-normalized target strength for demersal fish aggregations was derived from the relationship between the mean volume backscattering strength for the depth strata of trawl hauls and the weight per cubic meter of trawl catches. The geographical distribution of fish stocks in the 1994 survey area was investigated in relation to oceanographic conditions. The results obtained can be summarized as follows: 1. From the 1994 and 1995 survey data, the relationship between the mean volume backscattering strength (, dB) for the depth strata of trawl hauls and the weight (W, kg/$m^3$) per cubic meter of trawl catches was expressed by the following equation = - 32.8+ lOlog(W) The average weighted-target strength value at 50 kHz derived from this equation was .. 32.8 dB/kg. 2. In 1994 and 1995, both surveys showed a trend of decreasing fish abundance toward the southern area of the Cheju Island with high densities offish along the west coast ofth.e Tsushima Island. The highest demersal concentrations in the southern area of the CheJu Island appeared in bottom waters colder than $12^{\circ}C.$. . 3. From the results of combined bottom trawl and hydroacoustic surveys, the estunated fish densities in the southeastern area of the Cheju Island were 1.5488 x $10^-4$kg/$m^3$ in the 1994 surveyand 1.9498 x $10^-4$kg/$m^3$ in the 1995 survey, respectively.

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Heavy Snowfall Disaster Response using Multiple Satellite Imagery Information (다중 위성정보를 활용한 폭설재난 대응)

  • Kim, Seong Sam;Choi, Jae Won;Goo, Sin Hoi;Park, Young Jin
    • Journal of Korean Society for Geospatial Information Science
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    • v.20 no.4
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    • pp.135-143
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    • 2012
  • Remote sensing which observes repeatedly the whole Earth and GIS-based decision-making technology have been utilized widely in disaster management such as early warning monitoring, damage investigation, emergent rescue and response, rapid recovery etc. In addition, various countermeasures of national level to collect timely satellite imagery in emergency have been considered through the operation of a satellite with onboard multiple sensors as well as the practical joint use of satellite imagery by collaboration with space agencies of the world. In order to respond heavy snowfall disaster occurred on the east coast of the Korean Peninsula in February 2011, snow-covered regions were analyzed and detected in this study through NDSI(Normalized Difference Snow Index) considering reflectance of wavelength for MODIS sensor and change detection algorithm using satellite imagery collected from International Charter. We present the application case of National Disaster Management Institute(NDMI) which supported timely decision-making through GIS spatial analysis with various spatial data and snow cover map.

Improvement of TAOS data process

  • Lee, Dong-Wook;Byun, Yong-Ik;Chang, Seo-Won;Kim, Dae-Won;TAOS Team, TAOS Team
    • The Bulletin of The Korean Astronomical Society
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    • v.36 no.2
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    • pp.129.1-129.1
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    • 2011
  • We have applied an advanced multi-aperture indexing photometry and sophisticated de-trending method to existing Taiwanese-American Occultation Survey (TAOS) data sets. TAOS, a wide-field ($3^{\circ}{\times}3^{\circ}$) and rapid photometry (5Hz) survey, is designed to detect small objects in the Kuiper Belt. Since TAOS has fast and multiple exposures per zipper mode image, point spread function (PSF) varies in a given image. Selecting appropriate aperture among various size apertures allows us to reflect these variations in each light curve. The survey data turned out to contain various trends such as telescope vibration, CCD noise, and unstable local weather. We select multiple sets of stars using a hierarchical clustering algorithm in such a way that the light curves in each cluster show strong correlations between them. We then determine a primary trend (PT) per cluster using a weighted sum of the normalized light curves, and we use the constructed PTs to remove trends in individual light curves. After removing the trend, we can get each synthetic light curve of star that has much higher signal-to-noise ratio. We compare the efficiency of the synthetic light curves with the efficiency of light curves made by previous existing photometry pipelines. Our photometric method is able to restore subtle brightness variation that tends to be missed in conventional aperture photometric methods, and can be applied to other wide-field surveys suffering from PSF variations and trends. We are developing an analysis package for the next generation TAOS survey (TAOS II) based on the current experiments.

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The Effect of Pelvic Tilting and the Valsalva Maneuver on Electromyographic Activity of Erector Spinae During Squat Lifting and Lowering (골반경사방향과 발잘바기법이 물건 들어 올리기와 내리기 시 척추기립근의 활동전위에 마치는 영향)

  • Roh, Jung-Suk;Yi, Chung-Hwi;Chung, Bo-In;Lee, Young-Hee
    • Physical Therapy Korea
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    • v.5 no.1
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    • pp.30-43
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    • 1998
  • The purposes of this study were to examine the effect of two different pelvic alignments and the Valsalva maneuver on electromyographic (EMG) activity of the erector spinae during squat lifting and lowering, and to find an efficient method for squat lifting and lowering. Twenty hea1thy men in their twenties lifted and lowered loads using four different methods: 1) anterior pelvic tilt position with the Valsalva maneuver, 2) anterior pelvic tilt position without the Valsalva maneuver, 3) posterior pelvic tilt with the Valsalva maneuver, 4) posterior pelvic tilt without the Valsalva maneuver. The EMG activity of erector spinae was recorded during both lifting and lowering with each method. The EMG activity of each individual was normalized to EMG activity produced by muscle during maximal voluntary contraction. Two-way analysis of variance for repeated measures ($2{\times}2$) was used to analyze the effect of the two factors: 1) pelvic tilt position (anterior pelvic tilt, posterior pelvic tilt), 2) the Valsalva maneuver (with and without). Analysis was performed separately for the lifting and lowering. The results were as follows: 1) EMG activity of erector spinae was greater when the pelvis was tilted anteriorly than when the pelvis was tilted posteriorly during squat lifting and squat lowering. 2) There was no difference between EMG activity of erector spinae with the Valsalva maneuver and EMG activity of erector spinae without the Valsalva maneuver during squat lifting and squat lowering. These results suggest that the greater EMG activity of erector spinae with an anterior pelvic tilt position during squat lifting and squat lowering may ensure optimal muscular support for the spine while handling loads, but the Valsalva maneuver may have less effect on erector spinae.

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A laboratory and numerical study on the effect of geogrid-box method on bearing capacity of rock-soil slopes

  • Moradi, Gholam;Abdolmaleki, Arvin;Soltani, Parham;Ahmadvand, Masoud
    • Geomechanics and Engineering
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    • v.14 no.4
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    • pp.345-354
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    • 2018
  • Currently, layered geogrid method (LGM) is the commonly practiced technique for reinforcement of slopes. In this paper the geogrid-box method (GBM) is introduced as a new approach for reinforcement of rock-soil slopes. To achieve the objectives of this study, a laboratory setup was designed and the slopes without reinforcements and reinforced with LGM and GBM were tested under the loading of a circular footing. The effect of vertical spacing between geogrid layers and box thickness on normalized bearing capacity and failure mechanism of slopes was investigated. A series of 3D finite element analysis were also performed using ABAQUS software to supplement the results of the model tests. The results indicated that the load-settlement behavior and the ultimate bearing capacity of footing can be significantly improved by the inclusion of reinforcing geogrid in the soil. It was found that for the slopes reinforced with GBM, the displacement contours are widely distributed in the rock-soil mass underneath the footing in greater width and depth than that in the reinforced slope with LGM, which in turn results in higher bearing capacity. It was also established that by reducing the thickness of geogrid-boxes, the distribution and depth of displacement contours increases and a longer failure surface is developed, which suggests the enhanced bearing capacity of the slope. Based on the studied designs, the ultimate bearing capacity of the GBM-reinforced slope was found to be 11.16% higher than that of the slope reinforced with LGM. The results also indicated that, reinforcement of rock-soil slopes using GBM causes an improvement in the ultimate bearing capacity as high as 24.8 times more than that of the unreinforced slope.